Six cases of ACTH‐secreting tumors—which are shown to secrete additional hormonal substances—are reported. The high incidence of this phenomenon among our 15 observed cases with ACTH‐secreting tumor suggests that poly‐humoral secretion in ACTH‐secreting tumors is more common than previous reports would indicate. Although several of our cases are unique in that the particular combination of endocrine substances produced has not been previously reported, the tumor types (islet‐cell carcinoma, carcinoid, pheochromocytoma, oat‐cell carcinoma) are in general those which may be endocrine active in a variety of ways. Ectopic corticotrophin production arises principally in tumors of endocrine tissue, neuroendocrine tissue, endocrine dependent tissue and in oat‐cell carcinoma of the thorax. The possibility that oat‐cell carcinoma of the lung and mediastinum may be a more malignant variety of carcinoid tumor is not proven but should not yet be discounted.
The syndrome of multiple endocrine neoplasia type 2B (MEN 2B) resembles that of MEN 2A in that both include medullary carcinoma of the thyroid, pheochromocytoma, and autosomal dominant inheritance, but is distinct in that MEN 2B patients have neuromas of the mucous membranes. MEN2A has been linked to RBP3, D10S5, FNRB, D10S15, and D10Z1 near the centromere of chromosome 10. We examined linkage between MEN2B and RFLPs on chromosome 10 in all available members in two or three generations of 14 kindreds. The centromere marker D10Z1 was linked to MEN2B with a peak lod score of 5.42 at theta = 0.02. One possible recombinant was observed between D10Z1 and MEN2B. Multipoint analysis of RFLPs at FNRB, D10Z1, RBP3, and D10S15 gave a peak lod score of 7.12 at the midpoint between D10Z1 and RBP3 on the long arm (band q11). The most likely gene order FNRB-D10Z1-MEN2B was 27 times more likely than MEN2B-FNRB-D10Z1 and 31/2 times more likely than FNRB-MEN2B-D10Z1. Additional data will be required to establish the order of these loci with confidence.
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